Zhonghua yi xue za zhi · 2015
[Clinical analysis of 70 cases of polycythemia vera].
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Ren Yue, Fu Rong, Qu Wen, Ruan Erbao, Wang Xiaoming, Wang Guojin, Wu Yuhong, Liu Hong, Song Jia, Guan Jing, Xing Limin, Li Lijuan, Wang Huaquan, Shao Zonghong
Аннотация
OBJECTIVE: To analyze the clinical characteristics and laboratory data, treatment and prognosis of polycythemia vera (PV) and provide evidence for screening of high-risk population and looking for measures to reduce complecations.
METHODS: A retrospective study was performed among 70 patients with PV from May 2005 to May 2014, 43 males and 27 females, aged (56.6±13.1) to collect the data about characteristics, laboratory data, myelogram chromosome, karyotypes, BCR/ABL and JAK2 V617F genes, as well as lactate dehydrogenase (LDH) and so on.
RESULTS: At diagnosis, there were 42 cases (60.00%) had symptoms, 25 cases (35.71%) had thrombosis and embolism. Hemorrhage occurred in 3 cases (4.29%), splenomegaly in 48 cases (68.57%), hepatomegaly in 7 cases (10.00%). The average hemoglobin at diagnosis was 195.17 g/L, the white blood cells count was 10.12×10(9)/L, the platelet count was 295×10(9)//L. The chromosome karyotypes were all normal. The positive percentage of JAK2 V617F mutation was 87.69% (57/65). The disease outcomes were myelofibrosis for 3 paitents, death from ineffective treatment after transforming to myelofibrosis and then biphenotype acute leukemia for 1 patient, and death from cardiorespiratory failure for 2 patients. The level of erythropoietin in JAK2 V617F mutated group were significantly lower than those in wild-type JAK2 V617F group (P<0.05). The level of hemoglobin and platelet counts in JAK2 V617F mutated group were significantly higher than those in wild-type JAK2 V617F group (both P<0.05).
CONCLUSION: PV is one of meyloproliferation neoplasm, characterized by abnormally increasing blood cells, thrombosis and transforming to other myeloproliferative neoplasms.
METHODS: A retrospective study was performed among 70 patients with PV from May 2005 to May 2014, 43 males and 27 females, aged (56.6±13.1) to collect the data about characteristics, laboratory data, myelogram chromosome, karyotypes, BCR/ABL and JAK2 V617F genes, as well as lactate dehydrogenase (LDH) and so on.
RESULTS: At diagnosis, there were 42 cases (60.00%) had symptoms, 25 cases (35.71%) had thrombosis and embolism. Hemorrhage occurred in 3 cases (4.29%), splenomegaly in 48 cases (68.57%), hepatomegaly in 7 cases (10.00%). The average hemoglobin at diagnosis was 195.17 g/L, the white blood cells count was 10.12×10(9)/L, the platelet count was 295×10(9)//L. The chromosome karyotypes were all normal. The positive percentage of JAK2 V617F mutation was 87.69% (57/65). The disease outcomes were myelofibrosis for 3 paitents, death from ineffective treatment after transforming to myelofibrosis and then biphenotype acute leukemia for 1 patient, and death from cardiorespiratory failure for 2 patients. The level of erythropoietin in JAK2 V617F mutated group were significantly lower than those in wild-type JAK2 V617F group (P<0.05). The level of hemoglobin and platelet counts in JAK2 V617F mutated group were significantly higher than those in wild-type JAK2 V617F group (both P<0.05).
CONCLUSION: PV is one of meyloproliferation neoplasm, characterized by abnormally increasing blood cells, thrombosis and transforming to other myeloproliferative neoplasms.